Molecule

IgM (monoclonal)

Diagnostic paraprotein

Expression change
Present (obligate)
Evidence level
established
Targeted by
Ibrutinib (reduces), Rituximab (reduces but ineffective on symptoms)

Role in pathogenesis

Obligate diagnostic criterion. Kappa light chain predominance (15:1). Relationship to inflammation is the central unsolved mystery.

Targeting drugs (2)

DrugMechanismResponse rateLine
IbrutinibBTK inhibitorComplete in case reportsAlternative
RituximabAnti-CD20 (B-cell depletion)Ineffective on inflammationNot recommended

Sources (3)

A2Lipsker D et al. (2001) Lipsker diagnostic criteria for Schnitzler syndromeDOI ↗
J4Qiao J et al. (2022) Schnitzler and Schnitzler-like syndromes reviewPubMed ↗
A3de Koning HD et al. (2007) Landmark systematic review (94 patients)DOI ↗